Segmental Odontomaxillary Dysplasia: Unusual Tumoral Lesion.

IF 3.2 Q2 PATHOLOGY Head & Neck Pathology Pub Date : 2025-01-07 DOI:10.1007/s12105-024-01744-0

Fernanda Luiza Araújo de Lima Castro, Ana Cristina Tetzner, Roberta Rayra Martins-Chaves, Wagner Henriques de Castro, Ricardo Santiago Gomez

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Abstract

Introduction: Segmental Odontomaxillary Dysplasia (SOD) is a non-hereditary, unilateral developmental anomaly recently included in the WHO's classification of head and neck tumors.

Case presentation: Here, we report the case of an 8-year-old boy presenting with unilateral maxillary enlargement and pain without facial asymmetry. Computed tomography revealed a hypodense area in the maxillary bone with altered bone structure and osseous expansion. An incisional biopsy showed fibrous hyperplasia in the gingiva, dysplastic dentin, and reversal lines in the bone trabeculae. Following the diagnosis of SOD, the patient was referred for treatment.

Conclusion: To date, only 72 cases have been reported. SOD typically manifests in early childhood, with a male predominance. While the etiology remains unclear, mutations in the PIK3CA gene have been associated with its development. Further research is needed to better understand the disease and improve patient management.

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节段性牙颌瘤发育不良：不寻常的肿瘤病变。

节段性齿瘤腋窝发育不良（SOD）是一种非遗传性的单侧发育异常，最近被WHO纳入头颈部肿瘤分类。病例介绍：在这里，我们报告一个8岁的男孩，表现为单侧上颌增大和面部不对称的疼痛。计算机断层扫描显示上颌骨低密度区，骨结构改变和骨扩张。切口活检显示牙龈纤维增生，牙本质发育不良，骨小梁有反转线。诊断为SOD后，患者转介治疗。结论：迄今为止，仅报告了72例。超氧化物歧化酶通常表现在儿童早期，以男性为主。虽然病因尚不清楚，但PIK3CA基因突变与其发展有关。需要进一步的研究来更好地了解这种疾病并改善患者管理。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Head & Neck Pathology PATHOLOGY-

CiteScore

5.70

自引率

9.50%

发文量

期刊介绍： Head & Neck Pathology presents scholarly papers, reviews and symposia that cover the spectrum of human surgical pathology within the anatomic zones of the oral cavity, sinonasal tract, larynx, hypopharynx, salivary gland, ear and temporal bone, and neck. The journal publishes rapid developments in new diagnostic criteria, intraoperative consultation, immunohistochemical studies, molecular techniques, genetic analyses, diagnostic aids, experimental pathology, cytology, radiographic imaging, and application of uniform terminology to allow practitioners to continue to maintain and expand their knowledge in the subspecialty of head and neck pathology. Coverage of practical application to daily clinical practice is supported with proceedings and symposia from international societies and academies devoted to this field. Single-blind peer review The journal follows a single-blind review procedure, where the reviewers are aware of the names and affiliations of the authors, but the reviewer reports provided to authors are anonymous. Single-blind peer review is the traditional model of peer review that many reviewers are comfortable with, and it facilitates a dispassionate critique of a manuscript.